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. Author manuscript; available in PMC: 2015 Mar 1.
Published in final edited form as: Bone. 2013 Dec 31;60:246–251. doi: 10.1016/j.bone.2013.12.030

Figure 3. Hypothetical model representing pathogenetic mechanisms underlying Caffey Disease.

Figure 3

The R836C mutant proα1(I) chains may induce ER stress triggering an unfolded protein response leading to an upregulation of the COX2/PGE axis. Alternatively, the R836C mutant type I collagen fibrils may disrupt ligand interactions within the ECM resulting in signaling events that either directly or indirectly lead to elevated COX2/PGE signaling and altered resident cell function.